What’s Wrong with You?

   This is the question I am asked the most when people notice my chest scars or find out about my physical limitations, in a polite manner of course. And I will explain here in simple terms so all can fully understand. I had a surgical “correction” of the congenital heart defect, “Transposition of the Great Arteries.” TGA for short. That’s what they call it, more importantly it’s called the Mustard Procedure (Thanks to Dr William Mustard). It’s overwhelming to say let alone live with. Basically I was born with the two main arteries switched on top of my heart. So, in affect, my oxygenated blood went from my heart to my lungs and back to my heart while the unoxygenated blood circulated throughout my body. For that reason, I was born a blue baby and able to live outside my mother for a while due to the fact that when you are in vitro there is this hole between your heart chambers that allow your blood to flow unobstructed. Normally a short time after delivery this hole begins to close. But for me that closure would mean certain death. The first thing they had to do was to go up my femoral artery and surgically keep that hole open in order for my blood to comingle. Not the best solution but hey, from what I understand, oxygen is good for the brain.

This allowed me to be able to go home and become a little bigger and stronger before my open heart surgery. Seems the survival rate was much better if my body had time to develope a bit before some major alterations were made.

I had my open heart (Mustard) done in March of 1977, I was thirteen months old. This procedure had been developed in 1963 in the great country of Canada and eventually become the norm at the time, replacing the more complicated Senning procedure. With the Mustard, the surgeon creates a baffle from artificial material to redirect the flow of the oxygenated blood allowing my body to receive the nice clean blood from my lungs. This involves the opening of my heart to build the baffle which later in life will cause scar tissue to develop and in turn result in rhythm issues. More on that fun subject later.

So now that the baffle is done and I am put all back together there is still one major problem. My right side of my heart is doing the job normally done by the left side and vise versa. There inlies the problem for me later in life. Naturally and anatomically the heart was not designed for this. The side that had it easy from mother nature, circulating blood to the the lungs and back to the heart, is now forced to take on the burden of pumping the blood throughout my entire body. You see, a much different job. And as I get older the weaker it has become. Tired, worn out, exhausted, sick of working so hard, I guess would be a good description. That my friends is heart failure. But not your normal heart failure, nope it can’t be that cut and dry. You see the medicine developed for heart failure is designed for people with heart failure of the correct side of the heart that normally does the heavy lifting and from what i am told it is actually a different type of muscle. Who knew?

The initial outcome of my surgery was great. I grew real fast thanks to the good source of oxygen rich blood and I began to meet all the milestones of a “normal” baby. I was left with no permanent brain damage and no complications. I was lucky in the sense that TGA was the only defect I was born with because from what I understand some of us with TGA are also born with other heart abnormalities.

My childhood was, for the most part, normal. Besides the yearly visits to Philadelphia and wearing that damn holter monitor (24 hr. EKG recording device) that was the size of a boom box. Luckily my parents let me stay home from school to avoid the embarrassment and all the questions that would surely come. Only as I got into my early teens did the aesthetic part really start to bother me. I had this big scar on my chest which was embarrassing to me. And I started to have self esteem issues, as if teenage years aren’t hard enough to deal with. In my high school we were committed to taking swimming classes in order to graduate, that was a huge obstacle for me mentally. Also I had a slight lump on my chest from the year after my surgery. I had grown so fast that my chest plate at my sternum had buckled a bit. This was a major contention for me.

I would say I felt ok throughout my teen years and into my late twenties. At about the age of thirty did I notice a change in my abilities in my stamina. Mowing the grass in really hot weather and my performance in Kickboxing/MMA classes began to diminish. I knew the day would eventually come where I would start to have some issues, it had arrived. Not the greatest feeling in the world.

That’s when I began my research for a doctor or center that specialized in adults born with CHD and I found Penn University in collaboration with CHOP had such a program.

I had my consultation and all the test done. I was ok from a medical standpoint for someone with my condition and it was expected that my stamina would slowly begin to diminish over time but there was no answer as to how fast that would happen.

After the death of my parents and some personal stressful life events I began to feel worse. But, I pushed on and continued my visits to Philly.

I found out I needed a pacemaker due to my heart rate being in the toilet at night (27 bpm). I got that placed and what a blow to my moral. I viewed this as the beginning of the end, something I had feared and dreaded my entire life. But I needed it or I would wind up with a clot or stroke.

Then came the scariest moment so far, my heart wasn’t beating right and i could not stand without feeling like I was going to black out. I was taken to the hospital with a heart rate of 130 bpm. Once there and evaluated, they wanted to give me a drug to temporarily stop my heart to reset it to my normal rhythm. Umm, ok. The doctors told me there was a one percent chance it would not work and it would actually make my heart rate higher. Can you guess who belongs to the one percent group? Right! After the drug was injected I felt like I was going down a really steep roller coaster and I began to get tunnel vision. I remember them telling me to breath, all 10 of them packed in my emergency room with the defibrillator. Then my heart started back up and up and up it went. All the way to 230 bpm. It slowly came down over 5 minutes and stopped right back at 130. I was pissed. All that for what. I was admitted and given a medicine drip and overnight it broke my rhythm.

From that day forward I would have little episodes and constant PVC’s(skipped beats). I had a monitor hooked up that was through a cell phone so they could monitor me 24/7. My doctor called within a few days to let me know I was having periods of Atrial flutter. After consultation with an electrophysiologist it was determined I would need a cardiac ablation. The Atrial flutter was caused by all that scar tissue I had mentioned earlier. I didn’t hesitate because by this time I was having a hard time walking through the back yard or doing even the simplest things.

I had the ablation done almost two years ago from now and I have had not had any major episodes to date. I still have my PVC’s and take a ton of medication but I feel better than I did at that time. I’m tired all the time and my ejection fraction is in the toilet but it could be worse, it could be much worse. I am told that if my ejection fraction gets lower I will begin to be evaluated for a heart transplant. It is in my future but we just don’t know when, there’s no way to predict this.

Until then I live in the NOW, not yesterday, not tomorrow. Today is what I live for and enjoy.

I was born with a heart defect, so are 1 in 100 babies. I’m pretty sure that makes me more normal than I thought growing up. Please share my stories so others out there can feel normal too. 

One thought on “What’s Wrong with You?

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